Bleeding

Disorders
areta idarto
Inheritance Pattern
 Obligate carriers are: Possible carriers are:
- all daughters of a father
with hemophilia
- mothers of one son with
hemophilia and who have
at least one other family - all daughters of a carrier;
member with haemophilia - mothers of one son with
( a brother, maternal haemophilia but who do not
grandfather, uncle, have any other family
nephew, or cousin) members who have
hemophilia (or are carriers);
- mothers of one son with - sisters, mothers, maternal
haemophilia and who have grandmothers, aunts, ieces,
a family member who is a and female cousins of
known carrier of the carriers
haemophilia gene (a
mother, sister, materna
grandmother, aunt niece,
or cousin)
- mothers of two or more
 Bleeding Manifestation
• Hemarthrosis 70%-80%

• Knee: 45%

• Elbow : 30%

• Ankle : 15%

• Shoulder : 3%

• Wrist : 3%

• Hip : 2%

• Other : 2%

• Muscle / soft tissue: 10%-20%

• Other major bleeds: 5%-10%

 Life threatening bleeding

• Central nervous system (CNS): 5%

• Gastrointestinal (GI tract)

• Neck/throat
 Diagnostic evaluation

• Identify the potential cause of bleeding

• Platelet count, Bleeding Time (BT),

Prothrombin Time (PT), activated Partial
Thromboplastin time (APTT)

• Confirmation of diagnosis by factor assay

Diagnostic Test
 Hemophilia A
• Fresh whole blood
• 20-40% bleeding —> F VIII
• Fresh frozen plasma
• ABO, Rh
• 10-15 ml/kgBB (interval 8-12 ho)
• Cryopresipitate
• FVIII concentrate
 Hemophilia B

• Fresh whole blood

• Fresh Frozen plasma

• Cryopresipitate

• FIX concentrate
 Edukasi
• Menghindari trauma

• RICE

• Pemantauan efek samping terapi

• Pemeriksaan fungsi hate setiap 6 bulan

• Evaluasi tumbuh kembang

ITP
 ITP
• Immune Thrombocytopenia

• isolated thrombocytopenia due to an

unknown etiology

• formerly: idiopathic thrombocytopenic

purport

• now: restricted to a pathology with an

immunologycal cause
 ITP
• Bleeding disorder

• Acute-self limiting condition

• Recurrent

• Chronic autoimmune disorder

• Early destruction of platelet due to antibody

binding —> removal platelet by the
mononuclear phagocytic system
 ITP in children
• Acute form

• 80-90% acute bleeding episode

• resolving within a few days or weeks

• ending within 6 months ( 3 mo ), persistent 3-12 mo

• both sexes equally affected

• Peak occurrence 2-5 years of age

• history of viral, bacterial, or vaccination

• abrupt onset

• platelet < 20 x 109 /L

• Chronic ITP

• insidious onset, 1 y after dx

• predominance in female

• most affected age > 7 yo

• Recurrent

• occur in 1-4% children

• episode of thrombocytopeniaenia at interval > 3 months

• Familial ITP

• Rare disorder

• The nature is unclear

 Pathogenesis
• Increased rate of platelet destruction

• Bleeding occurs when platelet count fall < 10-

50 x 109/L

• Evidence of platelet associated IgG —>

immune complexes —> opsonisation —>
destruction of platelets (phagocytosis)

• Chronic ITP can be attributed to autoantibodies

directed against platelet (glicoprotein)
• secondary to autoimmune diseases: SLE,
Antiphospolipid syndrome, Evans syndrome

• Autoantibodies generated in response to

infection, vaccination, drugs (hapten
mechanism) —> directed against GPIIb/IIIa or
GPIb/V/IX
 Clinical Features
• Occurs within 1-3 weeks after an infectious
diseases (bacterial or non specific viral infection)

• Common first bleeding sign:

• bruising , petechiae —> platelet count < 20 x

109/L

• severe mucosal bleeding, hematuria, genital

bleeding —> platelet young < 10 x 109/L

• ICH occurs in 0.5-1%

• Apart from bleeding and thrombocytopenia —
> no other abnormal physical findings

• Bone marrow examination is indicated to

patients who fail to the treatment after 3-6
months
 Clinical Course
• Complete remission occurs in 80-90%

• 3-5% of cases develop chronic ITP mostly over 7

years of age

• Recurrent ITP occurs in 14% children with acute ITP

• ICH occurs in 0.5-1% hospitalised children and 1/3

of those cases are fatal

• Post splenectomy sepsis is a cause of death in

children with ITP
 Management
• Treatment is needed:

• Mucosal bleeding with PLT count < 20 x

109/L

• Minor bleeding with PLT count < 10 x 109/L

• Main therapy: corticosteroid

• Acute: prednison 2 mg/kgBW/d po ~ 7

days (tappering in 7 days)
• Chronic

• Corticosteroid

• Prednison 2 mg/kgBW/day/po or iv for 7

days (tappering in 7 days)

• Methylprednisolone 8-12 mg/kgBW/IV or

Dexamethasone 0.5-1.0 mg/kgBW/iv or po +
IVIF or PLT transfusion

• IVIG

• Cyclosporin

• Azathioprine
 Education

• Monitoring signs of bleeding

• Prevention and management of infection

thank you